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Bilateral Medullary Thyroid Carcinoma in a 3-Year-Old Female Patient with Multiple Endocrine Neoplasia 2A Syndrome

Abbas Al-Kurd1, David J Gross2, David Zangen3

  • 1Department of Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

European Thyroid Journal
|October 31, 2018
PubMed
Abstract

Insights

Prophylactic thyroidectomy in young patients with Multiple Endocrine Neoplasia (MEN) 2A and codon 634 mutations is crucial. Early screening and surgery are recommended due to the discovery of medullary thyroid carcinoma (MTC) in a 3-year-old patient.

Area of Science:

  • Endocrinology
  • Oncology
  • Genetics

Background:

  • Multiple Endocrine Neoplasia (MEN) 2A is an inherited disorder caused by RET proto-oncogene mutations.
  • It strongly predisposes patients to medullary thyroid carcinoma (MTC).
  • Current guidelines recommend prophylactic thyroidectomy for pediatric MEN 2A patients, with timing dependent on the specific mutation.

Purpose of the Study:

  • To evaluate the effectiveness of current guidelines for prophylactic thyroidectomy in MEN 2A.
  • To determine if the age threshold for thyroidectomy in codon 634 mutation carriers should be lowered.
  • To assess the utility of earlier serum calcitonin level evaluation.

Main Methods:

  • A case report of a 3-year-old female with MEN 2A (codon 634 mutation) undergoing prophylactic thyroidectomy.
  • Detailed reporting of preoperative diagnosis, surgical course, and postoperative findings.
  • Histopathologic analysis of the resected thyroid tissue.

Main Results:

  • Medullary thyroid carcinoma (MTC) was discovered bilaterally upon postoperative histopathologic examination.
  • A borderline increase in serum calcitonin levels was noted preoperatively.
  • The findings challenge the adequacy of the current prophylactic approach for this mutation.

Conclusions:

  • Current guidelines for MEN 2A may require revision.
  • Earlier calcitonin screening and prophylactic thyroidectomy are suggested for patients with codon 634 mutations.
  • This case highlights the aggressive nature of MTC in young patients with MEN 2A.
Keywords:
AgeGuidelinesIncidental carcinomaMedullary thyroid carcinomaMultiple endocrine neoplasia 2AProphylactic thyroidectomy

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